Studies on Progressive Hereditary Perceptive Deafness in A Family of 335 Members: I. Genetical and General Audiological Results
| Autor: | D.W. Odenthal, A. H. Van Bolhuis, E.H. Huizing |
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| Rok vydání: | 1966 |
| Předmět: | |
| Zdroj: | Acta Oto-Laryngologica. 61:35-41 |
| ISSN: | 1651-2251 0001-6489 |
| DOI: | 10.3109/00016486609127040 |
| Popis: | Progressive hereditary perceptive deafness has been studied in a family of 335 members in 5 generations with the following results: (1) The inheritance of the deafness in this family is of the autosomal-dominant type with complete penetrance. (2) The deafness is a symmetric pure perceptive hearing loss affecting high frequencies first, and most seriously. (3) The hearing impairment proceeds rapidly in the first 3 decades to 60-70 dB whereafter the progression slows down. (4) The progression of the hearing impairment follows a distinct pattern in which 5 characteristic stages are distinguished which are discussed in detail in a second article. (5) Characteristic stages of threshold loss correlate with typical speech audiometric curves. (6) Recruitment is present (modified monaural masking method), while difference limen of intensity is decreased (Sisi-test); perstimulatory adaptation is normal (tone decay test).Die progressive hereditare Innenohrschwerhorigkeit wurde untersucht in einer Familie von 335 Mit... |
| Databáze: | OpenAIRE |
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